Pulmonary arterial hypertension associated with pulmonary arteriovenous malformations and pulmonary veno-occlusive disease: A devastating combination.

Autor:	Zaaijer BM; University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Cardiology, the Netherlands., Duppen N; University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Cardiology, the Netherlands., Willemse BWM; University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Pulmonology and Allergology, the Netherlands., Verhagen MV; University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Radiology, the Netherlands., Roofthooft MTR; University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Cardiology, the Netherlands., Timens W; University of Groningen, University Medical Center Groningen, Department of Pathology and Medical Biology, the Netherlands., Berger RMF; University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Cardiology, the Netherlands., Douwes JM; University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Department of Pediatric Cardiology, the Netherlands.
Jazyk:	angličtina
Zdroj:	Respiratory medicine case reports [Respir Med Case Rep] 2021 Dec 01; Vol. 34, pp. 101564. Date of Electronic Publication: 2021 Dec 01 (Print Publication: 2021).
DOI:	10.1016/j.rmcr.2021.101564
Abstrakt:	We describe a case of an adolescent male with the rare combination of pulmonary arterial hypertension (PAH) and pulmonary arteriovenous malformations (PAVM's) without confirmed hereditary hemorrhagic telangiectasia (HHT). The patient showed clinical deterioration on standard vasodilator therapy, leading us to question our initial diagnosis. Post-mortem evaluation confirmed the presence of pulmonary veno-occlusive disease of which no conclusive signs were recognized at diagnostic work-up. This case demonstrates the heterogeneity in the diseases causing PAH and shows that an unexpected treatment response should alert the physician to question the original diagnosis. (© 2021 The Authors.)
Databáze:	MEDLINE
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